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2018 ; 7
(1
): 121-126
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Renal medullary carcinoma: a nearly fatal malignancy specifically affecting
patients with a so-called benign condition
#MMPMID29396817
Goenaga-Vázquez Y
; Colón G
; Barrios N
; Correa M
CEN Case Rep
2018[May]; 7
(1
): 121-126
PMID29396817
show ga
Renal medullary carcinoma (RMC) is a highly aggressive and rare malignancy found
almost exclusively in young patients with sickle cell trait (SCT). Metastatic
disease is commonly present at diagnosis. There is very limited experience
treating disseminated disease and the prognosis is dismal. We report the case of
a young 9-year-old boy with SCT, who presented with 4 months' progression of
abdominal pain, nausea and vomiting associated with cough spells, dysphagia, and
weight loss. Upon evaluation, he was underweight, pale, and in mild respiratory
distress. Cervical lymphadenopathy was evident and abdomen was diffusely tender.
A whole-body CT scan showed a left kidney lesion with associated cervical,
mediastinal, and retroperitoneal lymphadenopathy. Biopsy of a cervical lymph node
revealed metastatic RMC. Patient was started on combination chemotherapy with
paclitaxel, carboplatin, and gemcitabine followed by left adrenalectomy. In spite
of having advanced disease, our patient achieved an excellent response with a
progression-free survival of 17 months. Although SCT is thought to be a "benign"
condition, RMC is one devastating complication associated with it. Considering
its rarity, the near uniform associated fatality should prompt the question of
whether clinical practice should change regarding proper counseling of these
patients and raise awareness in the medical community.